메뉴 건너뛰기




Volumn 135, Issue 9, 2012, Pages 2699-2710

Morphological and functional reversal of phenotypes in a mouse model of Rett syndrome

Author keywords

breathing; MECP2; morphology; motor function; Rett syndrome

Indexed keywords

METHYL CPG BINDING PROTEIN 2; TAMOXIFEN;

EID: 84866403603     PISSN: 00068950     EISSN: 14602156     Source Type: Journal    
DOI: 10.1093/brain/aws096     Document Type: Article
Times cited : (117)

References (54)
  • 2
    • 0032830639 scopus 로고    scopus 로고
    • Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2
    • Amir RE, Van den Veyver IB, Wan M, Tran CQ, Francke U, Zoghbi HY. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nat Genet 1999; 23: 185-8.
    • (1999) Nat Genet , vol.23 , Issue.1 , pp. 85-88
    • Amir, R.E.1    Van Den Veyver, I.B.2    Wan, M.3    Tran, C.Q.4    Francke, U.5    Zoghbi, H.Y.6
  • 3
    • 27144528519 scopus 로고    scopus 로고
    • Can we relate MeCP2 deficiency to the structural and chemical abnormalities in the Rett brain?
    • Armstrong DD. Can we relate MeCP2 deficiency to the structural and chemical abnormalities in the Rett brain? Brain Dev 2005; 27 (Suppl. 1): S72-6.
    • (2005) Brain Dev , vol.27 , Issue.SUPPL. 1
    • Armstrong, D.D.1
  • 5
    • 0031761177 scopus 로고    scopus 로고
    • Decreased dendritic branching in frontal, motor and limbic cortex in Rett syndrome compared with tri-somy 21
    • Armstrong DD, Dunn K, Antalffy B. Decreased dendritic branching in frontal, motor and limbic cortex in Rett syndrome compared with tri-somy 21. J Neuropathol Exp Neurol 1998; 57: 1013-7.
    • (1998) J Neuropathol Exp Neurol , vol.57 , pp. 1013-1017
    • Armstrong, D.D.1    Dunn, K.2    Antalffy, B.3
  • 6
    • 29144440149 scopus 로고    scopus 로고
    • Hippocampal synaptic plasticity is impaired in the Mecp2-null mouse model of Rett syndrome
    • Asaka Y, Jugloff DG, Zhang L, Eubanks JH, Fitzsimonds RM. Hippocampal synaptic plasticity is impaired in the Mecp2-null mouse model of Rett syndrome. Neurobiol Dis 2006; 21: 217-27.
    • (2006) Neurobiol Dis , vol.21 , pp. 217-227
    • Asaka, Y.1    Jugloff, D.G.2    Zhang, L.3    Eubanks, J.H.4    Fitzsimonds, R.M.5
  • 7
    • 42149127627 scopus 로고    scopus 로고
    • Comparative study of brain morphology in Mecp2 mutant mouse models of Rett syndrome
    • Belichenko NP, Belichenko PV, Li HH, Mobley WC, Francke U. Comparative study of brain morphology in Mecp2 mutant mouse models of Rett syndrome. J Comp Neurol 2008; 508: 184-95.
    • (2008) J Comp Neurol , vol.508 , pp. 184-195
    • Belichenko, N.P.1    Belichenko, P.V.2    Li, H.H.3    Mobley, W.C.4    Francke, U.5
  • 8
    • 62149119249 scopus 로고    scopus 로고
    • Evidence for both neuronal cell autonomous and nonautonomous effects of methyl-CpG-binding protein 2 in the cerebral cortex of female mice with Mecp2 mutation
    • Belichenko NP, Belichenko PV, Mobley WC. Evidence for both neuronal cell autonomous and nonautonomous effects of methyl-CpG-binding protein 2 in the cerebral cortex of female mice with Mecp2 mutation. Neurobiol Dis 2009a; 34: 71-7.
    • (2009) Neurobiol Dis , vol.34 , pp. 71-77
    • Belichenko, N.P.1    Belichenko, P.V.2    Mobley, W.C.3
  • 9
    • 0030639079 scopus 로고    scopus 로고
    • Morphological study of neocortical areas in Rett syndrome
    • Belichenko PV, Hagberg B, Dahlstrom A. Morphological study of neocortical areas in Rett syndrome. Acta Neuropathol 1997; 93: 50-61.
    • (1997) Acta Neuropathol , vol.93 , pp. 50-61
    • Belichenko, P.V.1    Hagberg, B.2    Dahlstrom, A.3
  • 10
    • 0027963287 scopus 로고
    • Rett syndrome: 3-D confocal microscopy of cortical pyramidal dendrites and afferents
    • Belichenko PV, Oldfors A, Hagberg B, Dahlstrom A. Rett syndrome: 3-D confocal microscopy of cortical pyramidal dendrites and afferents. Neuroreport 1994; 5: 1509-13.
    • (1994) Neuroreport , vol.5 , pp. 1509-1513
    • Belichenko, P.V.1    Oldfors, A.2    Hagberg, B.3    Dahlstrom, A.4
  • 11
    • 65349122404 scopus 로고    scopus 로고
    • Widespread changes in dendritic and axonal morphology in Mecp2-mutant mouse models of Rett syndrome: Evidence for disruption of neuronal networks
    • Belichenko PV, Wright EE, Belichenko NP, Masliah E, Li HH, Mobley WC, et al. Widespread changes in dendritic and axonal morphology in Mecp2-mutant mouse models of Rett syndrome: evidence for disruption of neuronal networks. J Comp Neurol 2009b; 514: 240-58.
    • (2009) J Comp Neurol , vol.514 , pp. 240-258
    • Belichenko, P.V.1    Wright, E.E.2    Belichenko, N.P.3    Masliah, E.4    Li, H.H.5    Mobley, W.C.6
  • 12
    • 34748831111 scopus 로고    scopus 로고
    • MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number
    • Chao HT, Zoghbi HY, Rosenmund C. MeCP2 controls excitatory synaptic strength by regulating glutamatergic synapse number. Neuron 2007; 56: 58-65.
    • (2007) Neuron , vol.56 , pp. 58-65
    • Chao, H.T.1    Zoghbi, H.Y.2    Rosenmund, C.3
  • 14
    • 0035093830 scopus 로고    scopus 로고
    • Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice
    • Chen RZ, Akbarian S, Tudor M, Jaenisch R. Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice. Nat Genet 2001; 27: 327-31.
    • (2001) Nat Genet , vol.27 , pp. 327-331
    • Chen, R.Z.1    Akbarian, S.2    Tudor, M.3    Jaenisch, R.4
  • 15
    • 79955602167 scopus 로고    scopus 로고
    • Isolation of mecp2-null rett syndrome patient hips cells and isogenic controls through x-chromosome inactivation
    • Cheung AY, Horvath LM, Grafodatskaya D, Pasceri P, Weksberg R, Hotta A, et al. Isolation of MECP2-null Rett syndrome patient hiPS cells and isogenic controls through X-chromosome inactivation. Hum Mol Genet 2011; 20: 2103-15.
    • (2011) Hum Mol Genet , vol.20 , pp. 2103-2115
    • Cheung, A.Y.1    Horvath, L.M.2    Grafodatskaya, D.3    Pasceri, P.4    Weksberg, R.5    Hotta, A.6
  • 16
    • 24644490120 scopus 로고    scopus 로고
    • Reduced cortical activity due to a shift in the balance between excitation and inhibition in a mouse model of Rett syndrome
    • Dani VS, Chang Q, Maffei A, Turrigiano GG, Jaenisch R, Nelson SB. Reduced cortical activity due to a shift in the balance between excitation and inhibition in a mouse model of Rett syndrome. Proc Natl Acad Sci USA 2005; 102: 12560-5.
    • (2005) Proc Natl Acad Sci USA , vol.102 , pp. 12560-12565
    • Dani, V.S.1    Chang, Q.2    Maffei, A.3    Turrigiano, G.G.4    Jaenisch, R.5    Nelson, S.B.6
  • 18
    • 77949409962 scopus 로고    scopus 로고
    • Early postnatal behavioral changes in the mecp2-308 truncation mouse model of rett syndrome
    • De Filippis B, Ricceri L, Laviola G. Early postnatal behavioral changes in the Mecp2-308 truncation mouse model of Rett syndrome. Genes Brain Behav 2010; 9: 213-23.
    • (2010) Genes Brain Behav , vol.9 , pp. 213-223
    • De Filippis, B.1    Ricceri, L.2    Laviola, G.3
  • 19
  • 20
    • 33847266846 scopus 로고    scopus 로고
    • Reversal of neurological defects in a mouse model of Rett syndrome
    • Guy J, Gan J, Selfridge J, Cobb S, Bird A. Reversal of neurological defects in a mouse model of Rett syndrome. Science 2007; 315: 1143-7.
    • (2007) Science , vol.315 , pp. 1143-1147
    • Guy, J.1    Gan, J.2    Selfridge, J.3    Cobb, S.4    Bird, A.5
  • 21
    • 0035094767 scopus 로고    scopus 로고
    • A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome
    • Guy J, Hendrich B, Holmes M, Martin JE, Bird A. A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome. Nat Genet 2001; 27: 322-6.
    • (2001) Nat Genet , vol.27 , pp. 322-326
    • Guy, J.1    Hendrich, B.2    Holmes, M.3    Martin, J.E.4    Bird, A.5
  • 22
    • 77949470597 scopus 로고    scopus 로고
    • Abnormalities of cell packing density and dendritic complexity in the mecp2 a140v mouse model of rett syndromex-linked mental retardation
    • Jentarra GM, Olfers SL, Rice SG, Srivastava N, Homanics GE, Blue M, et al. Abnormalities of cell packing density and dendritic complexity in the MeCP2 A140V mouse model of Rett syndrome/X-linked mental retardation. BMC Neurosci 2010; 11: 19.
    • (2010) BMC Neurosci , vol.11 , pp. 19
    • Jentarra, G.M.1    Olfers, S.L.2    Rice, S.G.3    Srivastava, N.4    Homanics, G.E.5    Blue, M.6
  • 24
    • 69249232208 scopus 로고    scopus 로고
    • Breathing disorders in Rett syndrome: Progressive neurochemical dysfunction in the respiratory network after birth
    • Katz DM, Dutschmann M, Ramirez JM, Hilaire G. Breathing disorders in Rett syndrome: progressive neurochemical dysfunction in the respiratory network after birth. Respir Physiol Neurobiol 2009; 168: 101-8.
    • (2009) Respir Physiol Neurobiol , vol.168 , pp. 101-108
    • Katz, D.M.1    Dutschmann, M.2    Ramirez, J.M.3    Hilaire, G.4
  • 25
    • 7244243971 scopus 로고    scopus 로고
    • MECP2 is progressively expressed in post-migratory neurons and is involved in neuronal maturation rather than cell fate decisions
    • Kishi N, Macklis JD. MECP2 is progressively expressed in post-migratory neurons and is involved in neuronal maturation rather than cell fate decisions. Mol Cell Neurosci 2004; 27: 306-21.
    • (2004) Mol Cell Neurosci , vol.27 , pp. 306-321
    • Kishi, N.1    MacKlis, J.D.2
  • 26
    • 77951014800 scopus 로고    scopus 로고
    • Exogenous brain-derived neurotrophic factor rescues synaptic dysfunction in Mecp2-null mice
    • Kline DD, Ogier M, Kunze DL, Katz DM. Exogenous brain-derived neurotrophic factor rescues synaptic dysfunction in Mecp2-null mice. J Neurosci 2010; 30: 5303-10.
    • (2010) J Neurosci , vol.30 , pp. 5303-5310
    • Kline, D.D.1    Ogier, M.2    Kunze, D.L.3    Katz, D.M.4
  • 27
    • 45349089199 scopus 로고    scopus 로고
    • Environmental enrichment ameliorates a motor coordination deficit in a mouse model of Rett syndrome-Mecp2 gene dosage effects and BDNF expression
    • Kondo M, Gray LJ, Pelka GJ, Christodoulou J, Tam PP, Hannan AJ. Environmental enrichment ameliorates a motor coordination deficit in a mouse model of Rett syndrome-Mecp2 gene dosage effects and BDNF expression. Eur J Neurosci 2008; 27: 3342-50.
    • (2008) Eur J Neurosci , vol.27 , pp. 3342-3350
    • Kondo, M.1    Gray, L.J.2    Pelka, G.J.3    Christodoulou, J.4    Tam, P.P.5    Hannan, A.J.6
  • 28
    • 64449086698 scopus 로고    scopus 로고
    • Bdnf overexpression in hippocampal neurons prevents dendritic atrophy caused by Rett-associated MECP2 mutations
    • Larimore JL, Chapleau CA, Kudo S, Theibert A, Percy AK, Pozzo-Miller L. Bdnf overexpression in hippocampal neurons prevents dendritic atrophy caused by Rett-associated MECP2 mutations. Neurobiol Dis 2009; 34: 199-211.
    • (2009) Neurobiol Dis , vol.34 , pp. 199-211
    • Larimore, J.L.1    Chapleau, C.A.2    Kudo, S.3    Theibert, A.4    Percy, A.K.5    Pozzo-Miller, L.6
  • 31
    • 77953122395 scopus 로고    scopus 로고
    • Diminished Prevalence but preserved synaptic distribution of n-methyl-d-aspartate receptor sub-units in the methyl cpg binding protein 2(mecp2)-null mouse brain
    • Maliszewska-Cyna E, Bawa D, Eubanks JH. Diminished prevalence but preserved synaptic distribution of N-methyl-d-aspartate receptor sub-units in the methyl CpG binding protein 2(MeCP2)-null mouse brain. Neuroscience 2010; 168: 624-32.
    • (2010) Neuroscience , vol.168 , pp. 624-632
    • Maliszewska-Cyna, E.1    Bawa, D.2    Eubanks, J.H.3
  • 32
    • 78149488365 scopus 로고    scopus 로고
    • A Model for neural development and treatment of rett syndrome using human induced pluripotent stem cells
    • Marchetto MC, Carromeu C, Acab A, Yu D, Yeo GW, Mu Y, et al. A model for neural development and treatment of Rett syndrome using human induced pluripotent stem cells. Cell 2010; 143: 527-39.
    • (2010) Cell , vol.143 , pp. 527-539
    • Marchetto, M.C.1    Carromeu, C.2    Acab, A.3    Yu, D.4    Yeo, G.W.5    Mu, Y.6
  • 33
  • 34
  • 35
    • 38349055166 scopus 로고    scopus 로고
    • Early defects of GABAergic synapses in the brain stem of a MeCP2 mouse model of Rett syndrome
    • Medrihan L, Tantalaki E, Aramuni G, Sargsyan V, Dudanova I, Missler M, et al. Early defects of GABAergic synapses in the brain stem of a MeCP2 mouse model of Rett syndrome. J Neurophysiol 2008; 99: 112-21.
    • (2008) J Neurophysiol , vol.99 , pp. 112-121
    • Medrihan, L.1    Tantalaki, E.2    Aramuni, G.3    Sargsyan, V.4    Dudanova, I.5    Missler, M.6
  • 36
    • 66549124389 scopus 로고    scopus 로고
    • Remodelling of the respiratory network in a mouse model of Rett syndrome depends on brain-derived neurotrophic factor regulated slow calcium buffering
    • Mironov SL, Skorova E, Hartelt N, Mironova LA, Hasan MT, Kugler S. Remodelling of the respiratory network in a mouse model of Rett syndrome depends on brain-derived neurotrophic factor regulated slow calcium buffering. J Physiol 2009; 587 (Pt 11): 2473-85.
    • (2009) J Physiol , vol.587 , Issue.PART 11 , pp. 2473-2485
    • Mironov, S.L.1    Skorova, E.2    Hartelt, N.3    Mironova, L.A.4    Hasan, M.T.5    Kugler, S.6
  • 37
    • 30644479042 scopus 로고    scopus 로고
    • Learning and memory and synaptic plasticity are impaired in a mouse model of Rett syndrome
    • Moretti P, Levenson JM, Battaglia F, Atkinson R, Teague R, Antalffy B, et al. Learning and memory and synaptic plasticity are impaired in a mouse model of Rett syndrome. J Neurosci 2006; 26: 319-27.
    • (2006) J Neurosci , vol.26 , pp. 319-327
    • Moretti, P.1    Levenson, J.M.2    Battaglia, F.3    Atkinson, R.4    Teague, R.5    Antalffy, B.6
  • 38
    • 33646683567 scopus 로고    scopus 로고
    • MeCP2 dysfunction in Rett syndrome and related disorders
    • Moretti P, Zoghbi HY. MeCP2 dysfunction in Rett syndrome and related disorders. Curr Opin Genet Dev 2006; 16: 276-81.
    • (2006) Curr Opin Genet Dev , vol.16 , pp. 276-281
    • Moretti, P.1    Zoghbi, H.Y.2
  • 39
    • 38349044361 scopus 로고    scopus 로고
    • Activity-dependent suppression of miniature neurotransmission through the regulation of DNA methy-lation
    • Nelson ED, Kavalali ET, Monteggia LM. Activity-dependent suppression of miniature neurotransmission through the regulation of DNA methy-lation. J Neurosci 2008; 28: 395-406.
    • (2008) J Neurosci , vol.28 , pp. 395-406
    • Nelson, E.D.1    Kavalali, E.T.2    Monteggia, L.M.3
  • 41
    • 0026747147 scopus 로고
    • Motor symptoms of the Rett syndrome: Abnormal muscle tone, posture, locomotion and stereotyped movement
    • Nomura Y, Segawa M. Motor symptoms of the Rett syndrome: abnormal muscle tone, posture, locomotion and stereotyped movement. Brain Dev 1992; 14 (Suppl.): S21-8.
    • (1992) Brain Dev , vol.14 , Issue.SUPPL.
    • Nomura, Y.1    Segawa, M.2
  • 42
    • 35148840586 scopus 로고    scopus 로고
    • Brain-derived neurotrophic factor expression and respiratory function improve after ampakine treatment in a mouse model of Rett syndrome
    • Ogier M, Wang H, Hong E, Wang Q, Greenberg ME, Katz DM. Brain-derived neurotrophic factor expression and respiratory function improve after ampakine treatment in a mouse model of Rett syndrome. J Neurosci 2007; 27: 10912-7.
    • (2007) J Neurosci , vol.27 , pp. 10912-10917
    • Ogier, M.1    Wang, H.2    Hong, E.3    Wang, Q.4    Greenberg, M.E.5    Katz, D.M.6
  • 44
    • 33645124652 scopus 로고    scopus 로고
    • An altered neonatal behavioral phenotype in Mecp2 mutant mice
    • Picker JD, Yang R, Ricceri L, Berger-Sweeney J. An altered neonatal behavioral phenotype in Mecp2 mutant mice. Neuroreport 2006; 17: 541-4.
    • (2006) Neuroreport , vol.17 , pp. 541-544
    • Picker, J.D.1    Yang, R.2    Ricceri, L.3    Berger-Sweeney, J.4
  • 45
    • 78149411851 scopus 로고    scopus 로고
    • Progressive motor and respiratory metabolism deficits in post-weaning mecp2-null male mice
    • Pratte M, Panayotis N, Ghata A, Villard L, Roux JC. Progressive motor and respiratory metabolism deficits in post-weaning Mecp2-null male mice. Behav Brain Res 2011; 216: 313-20.
    • (2011) Behav Brain Res , vol.216 , pp. 313-320
    • Pratte, M.1    Panayotis, N.2    Ghata, A.3    Villard, L.4    Roux, J.C.5
  • 46
    • 69449090636 scopus 로고    scopus 로고
    • MECP2 isoform-specific vectors with regulated expression for Rett syndrome gene therapy
    • Rastegar M, Hotta A, Pasceri P, Makarem M, Cheung AY, Elliott S, et al. MECP2 isoform-specific vectors with regulated expression for Rett syndrome gene therapy. PLoS One 2009; 4: e6810.
    • (2009) PLoS One , vol.4
    • Rastegar, M.1    Hotta, A.2    Pasceri, P.3    Makarem, M.4    Cheung, A.Y.5    Elliott, S.6
  • 47
    • 34147198993 scopus 로고    scopus 로고
    • Treatment with desi-pramine improves breathing and survival in a mouse model for Rett syndrome
    • Roux JC, Dura E, Moncla A, Mancini J, Villard L. Treatment with desi-pramine improves breathing and survival in a mouse model for Rett syndrome. Eur J Neurosci 2007; 25: 1915-22.
    • (2007) Eur J Neurosci , vol.25 , pp. 1915-1922
    • Roux, J.C.1    Dura, E.2    Moncla, A.3    Mancini, J.4    Villard, L.5
  • 52
    • 30144441211 scopus 로고    scopus 로고
    • Mecp2 deficiency disrupts norepinephrine and respiratory systems in mice
    • Viemari JC, Roux JC, Tryba AK, Saywell V, Burnet H, Pena F, et al. Mecp2 deficiency disrupts norepinephrine and respiratory systems in mice. J Neurosci 2005; 25: 11521-30.
    • (2005) J Neurosci , vol.25 , pp. 11521-11530
    • Viemari, J.C.1    Roux, J.C.2    Tryba, A.K.3    Saywell, V.4    Burnet, H.5    Pena, F.6
  • 53
    • 79953167470 scopus 로고    scopus 로고
    • Synaptic Plasticity deficits in an experimental model of rett syndrome: Long-term potentiation saturation and its pharmacological reversal
    • Weng SM, McLeod F, Bailey ME, Cobb SR. Synaptic plasticity deficits in an experimental model of Rett syndrome: long-term potentiation saturation and its pharmacological reversal. Neuroscience 2011; 180: 314-21.
    • (2011) Neuroscience , vol.180 , pp. 314-321
    • Weng, S.M.1    McLeod, F.2    Bailey, M.E.3    Cobb, S.R.4
  • 54
    • 40849097804 scopus 로고    scopus 로고
    • The MeCP2-null mouse hippocampus displays altered basal inhibitory rhythms and is prone to hyperexcitability
    • Zhang L, He J, Jugloff DG, Eubanks JH. The MeCP2-null mouse hippocampus displays altered basal inhibitory rhythms and is prone to hyperexcitability. Hippocampus 2008; 18: 294-309.
    • (2008) Hippocampus , vol.18 , pp. 294-309
    • Zhang, L.1    He, J.2    Jugloff, D.G.3    Eubanks, J.H.4


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.